Sporadic inclusion body myositis: a continuing puzzle

@article{Needham2008SporadicIB,
  title={Sporadic inclusion body myositis: a continuing puzzle},
  author={Merrilee Needham and Frank L Mastaglia},
  journal={Neuromuscular Disorders},
  year={2008},
  volume={18},
  pages={6-16}
}

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References

SHOWING 1-10 OF 183 REFERENCES
Genetics of inclusion‐body myositis
TLDR
Current understanding of the contribution of genetic susceptibility factors to the development of sIBM is summarized in this review.
Sporadic inclusion body myositis—diagnosis, pathogenesis and therapeutic strategies
  • M. Dalakas
  • Biology, Medicine
    Nature Clinical Practice Neurology
  • 2006
TLDR
Emerging data imply that continuous upregulation of cytokines and major histocompatibility complex class I on the muscle fibers causes an endoplasmic reticulum stress response, resulting in intracellular accumulation of misfolded glycoproteins and activation of the transcription factor NFκB, leading to further cytokine activation.
Inclusion-body myositis
TLDR
It is proposed that the identified abnormal accumulation, misfolding, and aggregation of proteins, perhaps provoked by the aging milieu and aggravated by the oxidative stress, lead to the s-IBM-specific vacuolar degeneration and atrophy of muscle fibers.
HLA associations with inclusion body myositis
TLDR
The phenotype and phenotype data provide support for an autoimmune etiology for, and genetic predisposition to, IBM.
Inclusion body myositis in HIV-1 and HTLV-1 infected patients.
TLDR
It is concluded that IBM occurs in HIV-1 and HTLV-1 infected individuals and has a clinical, histological and immunological pattern identical to sporadic IBM in the non-retrovirally infected patients.
Molecular pathology and pathogenesis of inclusion‐body myositis
TLDR
The basic hypothesis is that overexpression of AβPP within the aging muscle fibers is an early upstream event causing a subsequent pathogenic cascade that leads to sporadic inclusion‐body myositis.
Proteomic Analysis of Inclusion Body Myositis
TLDR
Proteomics is validates proteomics as a powerful tool in the study of muscle disease and indicates a unique pattern of protein expression in IBM, which mainly serve as carriers for muscle contraction and other normal muscle functions.
Inclusion body myositis: Investigation of the mumps virus hypothesis by polymerase chain reaction
TLDR
The results therefore do not support the mumps hypothesis for IBM and the integrity of muscle RNA extracts was confirmed by PCR detection of constitutive Ableson tyrosine kinase mRNA.
Inclusion Body Myositis: Clonal Expansions of Muscle‐Infiltrating T Cells Persist Over Time
TLDR
Results show that in IBM there is clonal restriction of TCR expression in muscle‐infiltrating lymphocytes, and this indicates an important, continuous, antigen‐driven inflammatory reaction in IBM.
Inclusion body myositis-like phenotype induced by transgenic overexpression of βAPP in skeletal muscle
TLDR
Transgenic mice were derived in which selective overexpression of βAPP leads to the development of a subset of other histopathological and clinical features characteristic of IBM, including centric nuclei, inflammation, and deficiencies in motor performance, consistent with a pathogenic role for βAPP mismetabolism in human IBM.
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